Proteins involved in establishment and maintenance of imprinted methylation marks

Epigenetic phenomena are being increasingly recognized to play key roles in normal mammalian development and disease. This is exemplified by the process of genomic imprinting whereby despite identical DNA sequence, the two parental chromosomes are not equivalent and show either maternal- or paternal-specific expression at a subset of genes in the genome. These patterns are set up by differential DNA methylation marking at the imprinting control regions in male and female germ line. In this review, we discuss the specific mechanisms by which these methyl marks are established and then selectively maintained throughout pre-implantation development. Specifically, we discuss the recent findings of a critical role played by a KRAB zinc-finger protein ZFP57 and its co-factor KAP1/TRIM28 in mediating both processes.